tbuckley/cabot-search huggingface dataset:
PB2406: EFFECT OF PINK NOISE ON IMPROVING SLEEP QUALITY OF PATIENTS UNDERGOING HEMATOPOIETIC STEM CELL TRANSPLANTATION
Authors: Fangfang Wu, Zhenglan He, Aihua Sun
Journal: HemaSphere (2023)

Topic: 21. Stem cell transplantation - Experimental Background: At present, hematopoietic stem cell transplantation has become the main method for the treatment of malignant hematological diseases. High quality sleep can promote the treatment and rehabilitation of hematopoietic stem cell transplantation patients. Studies have shown that in the process of transplantation, a variety of reasons can make patients have bad emotions, affect the quality of sleep, and lead to sleep disorders.In addition, the results showed that 75% of people who were exposed to pink noise during nighttime and naps said they slept more safely listening to pink noise, increased their sleep security by 23% at night and increased their nap comfort by 45%.Therefore, we try to use pink noise to improve the sleep quality of patients undergoing hematopoietic stem cell transplantation Aims: To investigate the effect of pink noise on improving sleep quality of patients undergoing hematopoietic stem cell transplantation Methods: A total of 60 patients admitted to laminar flow ward for allogenic hematopoietic stem cell transplantation from 2022.1 to 2022.8 were randomly divided into control group (n = 30) and observation group (n = 30). The patients in the control group were given routine nursing measures before bedtime, and the observation group was given pink noise during sleep on the basis of the control group.The Pittsburgh Sleeping quality Index (PSQI) was used to compare the sleep quality between the two groups before and 2 weeks after intervention. Results: After 2 weeks of intervention, the sleep quality, sleep time, sleep time, sleep efficiency, sleep disorder, hypnotic drugs, daytime dysfunction score and total score (t ≥ 7.825) in the Pittsburgh sleep quality index scale (PSQI) in the observation group were significantly lower than those in the control group. Summary/Conclusion: Pink noise can effectively improve the sleep quality of patients undergoing hematopoietic stem cell transplantation. Keywords: Hematopoietic stem cell, Hematopoietic cell transplantation
PB2048: HEMATOLOGIC RECOVERY INDUCED BY AVATROMBOPAG IN SAA REFRACTORY OR INTOLERANT TO IMMUNOSUPPRESSIVE THERAPY COMBINED WITH ELTROMBOPAG
Authors: Xiaoqing Liu, Yuemin Gong, Yue LI, Yawen Zhang, Xinyu Zhang, Qiqiang Long, Guangsheng He, Jianyong LI
Journal: HemaSphere (2023)

Topic: 12. Bone marrow failure syndromes incl. PNH - Clinical Background: Intensive immunosuppressive therapy (IST) combined with eltrombopag contributes to improving hematopoiesis in patients with severe aplastic anemia (SAA). Eltrombopag, however, is associated with elevations in alanine aminotransferase and bilirubin. As a small molecule TPO receptor agonist, avatrombopag can be administered orally with food with no significant hepatotoxicity. Aims: We evaluated efficacy and safety of avatrombopag in patients with SAA refractory or intolerant to IST combined with eltrombopag. Methods: 20 SAA patients switched from eltrombopag to avatrombopag at a dose of 40 mg/day in Jiangsu Province Hospital and Second Hospital of Nanjing from October 2020 to February 2023. The hematologic response and safety were analyzed in this study. The data was collected by prospective registration in the Chinese Eastern Collaboration Group of Anemia (ChiCTR2100045895). Results: The median age was 20(3–74) years old. A total of four patients who did not respond to IST combined with eltrombopag within a median time of 13(4–16) months. 15 cases (75%) obtained at least one lineage hematologic response [2 cases (10%) of complete response (CR) and 13cases (65%) of partial response (PR)]. The median time to first response was 42(14–207) days. 13 (65%) patients had a platelet response, with a median increase in platelet count of 21×109/L (range: 11–109). 10 patients who had platelet transfusions requirement became transfusion-independent within a median avatrombopag duration of 35(11–80) days. 9 patients (45%) had erythroid responses, with a median increase in hemoglobin level of 18g/L (range: 12–47). 7 patients had been dependent on packed red cell transfusions, and 6 of them (86%) no longer required transfusions at a median time of 41(14–72) days after avatrombopag. A total of 12 patients (60%) had a neutrophil response, with a median increase of 1.2×109/L (range: 0.3–2.1). No avatrombopag-related grade 2 or more adverse events occurred during the administration, and no thrombotic events occurred. Summary/Conclusion: Our research indicated avatrombopag had certain efficacy and good tolerance in patients with SAA refractory or intolerant to IST combined with eltrombopag, which deserved further exploration.Keywords: Severe aplastic anemia, Immunosuppressive therapy
PB2169: LENALIDOMIDE, BORTEZOMIB AND DEXAMETHASONE COMBINATION THERAPY IN CONCOMITANT MYELODYSPLASTIC SYNDROME WITH ISOLATED 5Q DELETION AND MULTIPLE MYELOMA
Authors: Yu Han Koh, Christopher Dheepa, Lip Leong Chong, Eugene Fan, Hemalatha Shanmugam, Kiat Hoe Ong, Christian Aledia Gallardo
Journal: HemaSphere (2023)

Topic: 15. Myeloproliferative neoplasms - Biology & Translational Research Background: De-novo concurrent development of myelodysplastic syndrome (MDS) with isolated del(5q) and multiple myeloma (MM) is rare. Aims: We aim to report our experience with lenalidomide, bortezomib and dexamethasone therapy in a patient with MDS del(5q) and MM. Methods: A 73-year-old female presented with persistent neutropenia. Bone marrow analysis showed normocellular marrow with hypolobulated megakaryocytes. There was dysplasia in monopoiesis (CD11b-ht/56het), myelopoiesis (CD34+/13+/33+/117+), 7.7% plasma cell dyscrasia (CD45-/19-/56++/13dim/33dim/117dim) and a karyotype of 46, XX, del(5)(q13q33). FISH for MDS confirmed the deletion of 5q in 16% of the nuclei. Findings of plasma cell dyscrasia raised suspicion for clonal plasma cell disorder. Serum kappa free light chain level was 60.9mg/L, with free kappa/lambda ratio of 7.25. Serum protein electrophoresis was negative and urine immunofixation showed free kappa light chain band. She was asymptomatic with no evidence of end organ manifestations. A concurrent diagnosis of MDS del (5q) and MGUS was established. Over next 2 years, the patient had rising kappa free light chain to 104.9mg/L and worsening neutropenia. A repeat bone marrow showed plasma cells (39%), blasts (2%), and dysplastic changes in both erythroid and megakaryocytic lineages. Skeletal survey showed suspected lytic lesion over L3 vertebrae with no other end organ manifestations. She was treated for standard risk kappa free light chain myeloma and low-risk MDS with isolated del (5q). She tolerated lenalidomide 10 mg daily, bortezomib 1.3 mg/m2 and dexamethasone once weekly of a 28-day cycle, with no treatment related complications. Reassessment of bone marrow following 6 cycles of treatment showed a partial response for MM and complete response for MDS. She was offered autologous stem cell transplant for underlying myeloma which she declined. She completed 1 year of lenalidomide, bortezomib and dexamethasone therapy and is currently on maintenance therapy with lenalidomide with no neutropenia. Results: This case describes our experience of the standard three-drug induction therapy for this disease entity, which was not associated with significant hematologic toxicities. The role of transplant upfront remains under debate as we consider real-world factors such as treatment burden, long-term toxic effects, patient’s preferences, quality of life and cost burden. Keywords: Myelodysplastic syndrome, Multiple myeloma
PB2302: A TRNA-DERIVED SMALL RNAS EXPRESSION SIGNATURE AS A PREDICTIVE AND PROGNOSTIC BIOMARKER IN DIFFUSE LARGE B CELL LYMPHOMA
Authors: Jun Rao, Lin Xia, Qiong LI, Naya MA, Xinlei LI, Lidan Zhu, Dezhi Huang, Jiali LI, Pan Zhao, Yunjing Zeng, Sha Zhou, Huanping Guo, Jing Xia, Shifeng Lou, Fangyi Fan, Jin Wei, LI Gao, XI Zhang
Journal: HemaSphere (2023)

Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical Background: Diffuse large B cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma, and has a highly aggressive and heterogeneous clinical course. The identification of novel predictive molecular biomarkers remains an unmet clinical need. Aims: To construct a new prognostic model for DLBCL based on tsRNAs. Methods: Serum samples from 10 DLBCL patients and 5 healthy controls were investigated using non-coding RNA sequencing. Six tsRNAs were selected using random forest and principal component analysis, a 6-tsRNA-based classifier was established using cox proportional hazards modelling with a leave-one-out (LOO) cross-validation strategy, RT-qPCR was used to measure tsRNA expression in internal testing set, external testing cohort and 35 healthy controls. Results: We found that tsRNAs were sensitive biomarkers in both early detection, drug response, and prognosis. Specifically, high-risk patients stratified using the classifier had significantly lower overall survival (Hazard ratio: 6.657, 95%CI 2.827-15.68, P=0.0006) compared with low-risk patients. The 6-tsRNA-based classifier showed good performance in the internal testing set (n=100) and the independent external set (n=160). High risk was significantly correlated with the patient’s disease histology subtype, stage, and International Prognostic Index (IPI) scores. When integrating the classifier into IPI model, the novel system showed a superior prognostic prediction value and stability compared with IPI and 6-tsRNA-based classifier. In addition, dynamic classifier analysis demonstrated that patients with 1.06 drops after one cycle (early molecular response [EMR]) had better treatment responses and a superior prognosis. The 6-tsRNA-based signature enabled precise discrimination between healthy controls and DLBCL with AUC 0.882 (95%CI 0.826-0.939). Summary/Conclusion: Our 6-tsRNA expression-based risk classifier may make contributions to individual prognosis prediction and treatment decision-making. Keywords: Prognosis, DLBCL, Prediction
PB2470: VENO-OCCLUSIVE DISEASE (VOD)/SINUSOIDAL OBSTRUCTION SYNDROME (SOS) INCIDENCE AND TREATMENT TRENDS INSIGHTS FROM LATEST SYSTEMATIC REVIEW OF CASE REPORTS (2018-2023)
Authors: Saurabh Aggarwal, Sushil Kumar, Amrit Bela, Ozlem Topaloglu
Journal: HemaSphere (2023)

Topic: 22. Stem cell transplantation - Clinical Background: Veno-occlusive disease (VOD), also called sinusoidal obstruction syndrome (SOS), is a potentially life-threatening complication of hematopoietic stem cell transplantation (HSCT) conditioning or high-dose nontransplant chemotherapy. Aims: To understand incidence and treatment trends for Veno-Occlusive Disease (VOD) using latest published case reports. Methods: PubMed, Medline and NOVEL-VOD databases were searched for English language publications dated January 1, 2018 to December 31, 2022. Case reports were selected for inclusion if publications reported incidence or treatment for VOD (also called SOS-Sinusoidal Obstruction Syndrome). Selected publications were tagged as epidemiology, treatment trends, defibortide treated, pulmonary or hepatic VOD. Each category of case reports were reviewed to develop trends, insights and learnings for latest trends in VOD. Results: A total of 347 full text records on VOD/SOS were identified, of which 46 included case reports. The individual case reports and case services cumulatively reported on VOD incidence or treatment in 99 patients. In 30 patients, the incidence of VOD was induced due to chemotherapies such as pyrrolizidine alkaloids, thioguanine and oxaliplatin. Defibrotide was used in 12 patients. Pulmonary VOD was reported in 14 case reports with most mentioning challenges in diagnosis; hepatic VOD was reported in 13 publications. In 11 case reports, severe or very severe forms of VOD were reported due to chemotherapy and/or conditioning regimens for transplant. Treatment related case reports mention early diagnosis and timely treatment of VOD led to improved outcomes. Summary/Conclusion: Latest case reports provide potentially useful real-world evidence on incidence and treatment trends for rare disease such as VOD. Keywords: Veno-occlusive disease
PB2058: SCREENING FOR ANTINEUTROPHIL ANTIBODIES IN PATIENTS WITH SEVERE CHRONIC NEUTROPENIA AT ALEXANDRIA UNIVERSITY CHILDREN’S HOSPITAL
Authors: Ahmed Werby, Mostafa Salama, Hanan Nazir, Neveen Mikhael, Yasmine El Chazli
Journal: HemaSphere (2023)

Topic: 12. Bone marrow failure syndromes incl. PNH - Clinical Background: Neutropenia is defined as an absolute neutrophil count (ANC) <1.5 ×109/L. Neutropenia is considered “severe” when the ANC is < 0.5 ×109/L and “chronic” when it lasts longer than 3 months. There are several causes of isolated severe chronic neutropenia (SCN), including congenital and acquired causes like autoimmune neutropenia (AIN). Aims: To screen for antineutrophil antibodies in children with SCN in our center. Methods: This cross-sectional study included all SCN patients who were following at the hematology clinic of Alexandria University Children’s Hospital, Alexandria, Egypt. The demographic, initial clinical, and laboratory data were collected. We tested for antineutrophil antibodies using the LABscreenTM Multi kits (Thermo Fischer) using a Luminex technique to detect the presence of human neutrophil antibodies (HNA) specific antibodies in patient’s serum. Results: A total of 27 patients were included; sixteen (59.3%) patients were males. The patients’ age ranged from 11 to 218 months, and the mean age at the onset of neutropenia was 21.1 months. The majority of patients (n=26, 96.3%) presented with an infection, with a median ANC of 0.7 ×109/L and a median absolute monocytic count of 0.4 ×109/L. Overall, at least one antineutrophil antibody was detected in 17 patients (63%) of individuals. The most prevalent antibodies were against HNA-1a (n=7), and HNA-1c (n=5), while the least prevalent were HNA-a2 and HNA-4a (each in only one patient). The diagnosis of congenital neutropenia was confirmed in 8 patients (either severe congenital neutropenia or cyclic neutropenia), of whom 5 had at least one detectable antibody (62.5%), while 12 out of 19 patients with no evidence of congenital neutropenia had at least one detectable antibody (52%). Summary/Conclusion: Antineutrophil antibody detection was common among children with SCN and was not specific to patients suspected to have immune/idiopathic neutropenia, as it was also found in patients with proven congenital neutropenia. Detecting antineutrophil antibodies may not be enough to exclude a diagnosis of congenital neutropenia. Repeating the test over time and using different techniques of measuring antineutrophil antibodies may be warranted to confirm the diagnosis of autoimmune neutropenia in suspected patients. Keywords: Neutropenia, Anti-neutrophil antibody, Severe congenital neutropenia, Severe chronic neutropenia
PB2262: PROGNOSTIC FACTORS IN PATIENTS WITH RELAPSED/REFRACTORY HODGKINN LYMPHOMA
Authors: Kmira Zahra, Rihab Ben Sghaier, Monia Guermazi, Nesrine Ben Sayed, Ouahchi Ines, Monia Zaier, Yosra Ben Youssef, Haifa Regaieg, Abderrahim Khelif
Journal: HemaSphere (2023)

Topic: 17. Hodgkin lymphoma - Clinical Background: Hodgkin’s Lymphoma (HL) has been commonly recognized as a chemo-sensitive and potentially curable neoplastic disease. However, some patients are refractory or relapse after frontline therapy, and their prognosis is severely impacted by a potential outcome predictors. Aims: This study’s aim was to identify potential predictors of worse overall survival (OS) and event free survival (EFS) in patients affected by R/R HL treated with salvage therapy. Methods: We included R/R HL patients diagnosed at the hospital of Farhat Hached Sousse, between 2014 and 2020. The impact on overall survival (OS) and on event free survival (EFS) of the following prognostic parameters was studied: age of ≥ 45 years old, extra lymphatic infiltration, presence of B symptoms, lymph node involvement >3 sites, bulky disease, serum albumin<30g/dl, advanced stage (III -IV), and chemo sensitivity at the second salvage therapeutic regimen. Survival curves were compared with Log-rank test, Cox proportional hazards test was used for multivariate analysis. Results: Of the 41 patients analyzed, 12 (29%) had early-stage and 29(71%) had advanced-stage (IIB or higher) HL. Median age was 34 years [18-67], 21 (51%) of patients were male, 23 (56%) presented with B-symptoms and 13 (31, 7%) had bulky disease. Salvage therapy was indicated for relapsed HL in 7 patients (17%), and for HL refractory to first-line CT in 34 patients (83%). After a median follow up of 48 months the 3-year OS and PFS for the whole population were 63% and 10%, respectively. In univariate analysis lymph node involvement >3 sites, B symptoms, absence of chemo sensitivity at the second salvage therapeutic regimen, were associated with a shorter OS (p<0.05), while serum albumin <30g/l and advanced age, were associated with worse EFS (p<0.05). In multivariate analysis, no prognostic factor was correlated with OS and EFS and this can be explained by the small number of our patients. Summary/Conclusion: We demonstrated that absence of chemo sensitivity at the second salvage therapeutic regimen was associated with poor OS indicating the impact of choosing the best salvage regimen to achieve promptly the complete remission. Keywords: Hodgkin’s lymphoma
PB2192: JAK2 V617F ALLELE BURDEN AND THROMBOTIC EVENTS IN PATIENTS WITH JAK2 V617F POSITIVE POLYCYTHEMIA VERA OR ESSENTIAL THROMBOCYTHEMIA AT HIGH RISK OF THROMBOSIS
Authors: Yun Hui Hwang, Sung Hwa Bae, A-Jin Lee, Min Kyoung Kim
Journal: HemaSphere (2023)

Topic: 16. Myeloproliferative neoplasms - Clinical Background: The goal of therapy of polycythemia vera (PV) and essential thrombocythemia (ET) is primarily to prevent vascular events that are the leading cause of morbidity and mortality. Patients older than 60 years or with a previous history of thrombotic complications are considered at high risk of thrombosis. Aims: The aim of this study was to explore the incidence of thrombotic events and long-term outcomes in high-risk patients with JAK2 V617F positive PV or ET. Methods: We retrospectively evaluated the clinical outcomes of JAK2 V617F positive PV and ET at a high risk of thrombosis. The patients older than 60 years of age and/or had a history of thrombosis at diagnosis among patients with JAK2 V617F positive PV or ET from Daegu Catholic University Hospital between 2010 and 2021 were included in this study. Results: A total of 112 patents, median age 73 years, with JAK2 V617F positive PV (n=50) or ET (n=62) were included. One hundred two patients were over 60 years old and 46 patients had a history of thrombosis or thrombotic event at diagnosis. All patients were treated with cytoreductive drugs. The cumulative incidence of thrombosis at 7 years was 29.9%, corresponding to 32.7% in PV and 29.2% in ET patients. Arterial thrombosis (93.8%) predominated in both diseases. Forty-five patients died during the follow-up period. Median overall survival was 125.1 months (95% CI 85.8-164.4). Median thrombosis-free survival was 86 months (95% CI 68.4-103.6). Older age (≥75 years old) and a higher JAK2 V617F allele burden (≥25%) were associated with poor thrombosis-free survival. Estimated rates of thrombosis-free survival at 7 years were 73.9% in the patients with lower JAK2 V617F allele burden (<25%) and 44.1% in the patients with higher JAK2 V617F allele burden (≥25%) (p=0.015). Eleven patients (9.7%) had secondary myelofibrosis, and one patient developed acute myeloid leukemia. Summary/Conclusion: A higher JAK2 V617F allele burden is associated with a higher risk of thrombotic events in patients with high-risk PV and ET. Current therapy remains suboptimal, with ongoing risk for thrombosis and death in high-risk PV and ET patients with higher JAK2 V617F allele burden. A novel approach will be needed in this group. Keywords: Essential Thrombocytemia, Polycythemia vera, Thromboembolic events
PB2309: OUTCOMES OF 22 PATIENTS WITH RELAPSED OR REFRACTORY PRIMARY MEDIASTINAL LARGE B-CELL LYMPHOMA: SINGLE INSTITUTE EXPERIENCE
Authors: Gayane Tumyan, Arman Arakelyan, Anastasia Semenova, Inga Ilyasova, Petrova Galina, Maria Kichigina, Vladislav Kirsanov, Oksana Trofimova, Valeria Shpirko, Danila Lifentsev, Evgenia Paramonova, Daria Tupitsyna, Yulia Kluchagina, Dzhelil Shevcetovich
Journal: HemaSphere (2023)

Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical Background: Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive non-Hodgkin lymphoma with poor outcomes in patients with relapsed/refractory (R/R) disease. The standard of care for eligible patients (pts) with R/R PMBСL is salvage chemotherapy followed by high dose chemotherapy with autologous stem cell transplantation (ASCT). Unfortunately, in a significant part of pts, the disease is not sensitive to second-line therapy. Aims: This analysis was performed to determine the outcomes of pts with R/R PMBCL, who were treated with new therapeutic strategies - salvage chemotherapy in combination with programmed death-1 antibody (Nivolumab) + anti-CD30 antibody-drug conjugate (Brentuximab vedotin). Methods: A total of 22 pts (there were 17 females, median age 35, range 20-48 year) had relapsed (18 pts) or was refractory (4 pts) to one prior anthracycline-based chemotherapy regimen (R-CHOP, R-DA-EPOCH), 5 (23%) previously received radiation therapy. Patients typically received 2–3 cycles of platinum-based or other salvage chemotherapy (R-СT) such as R-DHAP (41%), R-ICE (45%), R-BeGeV (32%), R-B (27%). 5 pts (23%) was treated with Nivo+R-СT (median - 2 cycles; range 1-9), 14 (64%) - with Nivo+Bv+R-СT (median - 3 cycles; range, 1-5). After 2-3 cycles pts underwent disease reassessment by PET/CT scan. Patients who had a CR or PR proceeded to stem cell mobilization and ASCT, those who had evidence of less than PR or progressive disease could be treated at the next salvage regimen. Median line of salvage therapy - 3 (range 1-7). Results: With a median follow-up of 28 months, the objective response rate (ORR) was 77% (17 pts), 55% (12 pts) achieving CR. Seven responders (47%) received subsequent HCT (6 auto-HCT and 1 allo-HCT), 6 (27%) - underwent radiation consolidation. The 2-year PFS rate after diagnosis R/R was 37% (median 10 months), and the 2-year OS rate was 65% (median was not reached). Summary/Conclusion: Previously published retrospective results suggest that the outcomes of patients with R/R PMBСL were poor: the ORR to salvage chemotherapy was approximately 25%, and 2-year OS after diagnosis of relapse was only 15% [Kuruvilla J, 2008]. Our results indicate that combination of Nivo+Bv+R-СT demonstrated a high and prolong ORR, may be synergistic and serving as a potential bridge to effective consolidative therapies. Keywords: Lymphoma therapy, Relapsed lymphoma, Non-Hodgkin’s lymphoma, B cell lymphoma